This article provides a terrific summary of the most urgent issues with regard to caring for children with PALF; this article provides helpful information for diagnosis, and management, as well as information on pathophysiology, and associated outcomes.
The article makes a number of recommendations for testing/treatment -here are a few of them:
While the initial testing does list ferritin, it does not list soluble IL2R as an early test (listed lower in Table 4 under section of hepatic encephalopathy). My colleagues at Emory who specialize in liver transplantation have frequently recommended this test early in the evaluation of severe liver disease/acute liver failure (ALF) as a potential marker of HLH and immune dysregulation.
With regard to coagulopathy: “Coagulopathy secondary to vitamin K deficiency should rapidly correct following appropriate repletion. Notably, if coagulopathy persists, efforts to “correct” abnormal coagulation profiles with fresh frozen plasma or other pro-coagulation products should generally be avoided” [in the absence of bleeding or need for invasive procedure].
Initial IV Fluids: ” In the absence of the need for volume resuscitation, total intravenous fluids should initially be restricted to around 90% of maintenance fluids to avoid overhydration. Initial fluids should be similar to hypertonic glucose (D10) one-half normal saline and supplemented with 15 mEq of potassium (K+)/L.”
Nutritional support: “Patients with PALF are likely catabolic and require more calories than basal needs. Enteral feeding is often preferred over total parenteral nutrition (TPN), and both naso-gastric or naso-jejunal feeds should be considered before TPN.”
Neonatal ALF: “GALD results from an intrauterine alloimmune liver injury and is suspected to be the single most common cause of neonatal acute liver failure… Characteristic clinical features of GALD include an ALF presentation usually at birth and almost always in the first days of life. The majority (70–90%) of affected infants are born premature and a history of maternal sibling death is common. Timely exchange transfusion and high-dose intravenous immunoglobulin (IVIG) is the preferred treatment to remove offending antibodies and block their action, including activation of complement (88,91). The mechanism of GALD places subsequent pregnancies at risk, and intrapartum IVIG should be used to prevent recurrences.”
Table 8 list common medications implicated in PALF. “Acetaminophen (APAP) …remains the most common cause of DILI, and is the most common identified cause of ALF in children.”
Time to Adjust the Knowledge Doubling Curve in Hepatology In this post, AASLD expert guidance: for platelets in the setting of cirrhosis: “Given the low risk of bleeding of many common procedures, potential risks of platelet transfusion, lack of evidence that elevating the platelet count reduces bleeding risk, and ability to use effective interventions, including transfusion and hemostasis if bleeding occurs, it is reasonable to perform both low‐ and high‐risk procedures without prophylactically correcting the platelet count.” For INR in setting of cirrhosis: “The INR should not be used to gauge procedural bleeding risk in patients with cirrhosis who are not taking vitamin K antagonists (VKAs)…Measures aimed at reducing the INR are not recommended before procedures in patients with cirrhosis who are not taking VKAs…FFP transfusion before procedures is associated with risks and no proven benefits.”
Figure 3: Etiology of acute liver failure in children. (A) Etiology for 1144 children from the Pediatric Acute Liver Failure Study Group (PALFSG) 1999–2014. (B) Final diagnosis by age (note: figure B includes information on only 985 participants)
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